Increase In Cyst Size Research Articles

Atypical arachnoid cyst with progression: a case report

A 26-year-old female patient applied to the outpatient clinic in 2018 due to pain that had been present for a long time (16 years) and had recently spread from the top of the head to the nape of the neck. It was learned from the patient's anamnesis that her pain was relieved with analgesics. Although no pathological findings were found in the neurological examination, brain MRI was requested to reveal possible intracranial pathological conditions. In the MRI images taken, a cystic mass with a lobulated contour and thin septa of 24x31x40 mm and similar intensity to CSF was detected in the right parasagittal area posteriorly at the vertex level. The patient's control brain MRI images 3 months later showed no increase in cyst size. However, the patient's anamnesis, which came for a control in 2022, showed that his current complaints and headaches had increased. The MR images showed that the existing cyst had reached a size of approximately 60x35 mm. Medical treatment was initially planned for the patient (Acetazolamide, 750 mg/day). The MR taken one month later showed that there was no reduction in the cyst size and the optic discs were slightly swollen in the fundus examination. Considering the cyst growth, the patient's failure to benefit from medical treatment and the swelling noted in the optic discs, the patient was fitted with a cystoperitoneal shunt. The patient's early postoperative tomography image showed a significant reduction in the cyst (50.15x25.66) and a slightly swollen optic disc image with blurred borders was detected in the fundus examination. The patient's complaints regressed, and in the radiological images it was determined that the cyst continued to shrink [(45.8x23.19 mm) and (41.1x20.3 mm)], the shunt was in place, and she had no clinical complaints. As a result, it was argued that patients with atypically located arachnoid cysts, as in this case, should be taken under close clinical and radiological follow-up, and surgical treatment methods should definitely be considered in patients who are found to have an increase in cyst size and clinical findings during this follow-up and who do not respond despite medical treatment.

Epididymal cysts in children: frequency, clinical characteristics, and management strategies.

Epididymal cysts (ECs) are uncommon in the pediatric population. The objective of this study was to evaluate the frequency, clinical characteristics, and management strategies of ECs in children. We performed a retrospective review of pediatric scrotal ultrasounds between January 2014 and August 2022 to identify children with ECs. One hundred and forty-three children boys were found to have ECs, with 95 being pre-pubertal and 48 post-pubertal. The age of the patients ranged from 1 day to 18 years, with a mean age of 10.64 ± 4.55 years. The size of the cysts varied from 2 mm to 35 mm. The most common comorbidities observed were hydrocele, testicular microlithiasis and varicocele. The majority of ECs were detected through routine physical examination. Conservative management was employed for all patients, except for one who required surgical excision. Resolution of ECs occurred in 12 patients, while a reduction in cyst size was observed in 6 cases. Conversely, 2 patients experienced an increase in cyst size, and 6 patients exhibited an increase in cyst number during the follow-up period. Conservative management is the preferred approach for the majority of cases, with surgical intervention reserved for specific instances.

Dermoid cyst management and outcomes: a review of over 1000 cases at a single institution

BackgroundMature cystic teratomas represent nearly 60% of benign ovarian neoplasms across all age groups. We aim to update existing descriptive studies of ovarian teratomas including the epidemiology, rate of torsion/malignancy, and treatment modalities in a large modern cohort of patients. Study DesignThis is a retrospective cross-sectional study of all pathology-confirmed cases of ovarian teratoma who underwent surgery at one tertiary care institution from 2004–2015. Patient demographics, ovarian cyst characteristics, surgical approach and timing, rate of spillage, and surgical complications were examined. Results1,054 cases of ovarian teratoma were identified during the study period. There were 113 cases of bilateral teratoma (10.7%). Mean age at diagnosis was 38 years. Average cyst size was 6.26cm. The overall rate of torsion was 5.6%, with a higher rate of torsion with increasing cyst size. Over 70% of cases were treated with minimally invasive surgery, which was associated with decreased perioperative complications but an increased risk of cyst spillage. Among 394 patients with cyst spillage, only one developed chemical peritonitis. The malignant transformation rate of mature cystic teratoma in this cohort was 1.1%. This cohort included 100 pregnant women with mature teratoma. Pregnant patients were more likely to have minimally invasive surgery in the 1st trimester and more likely to undergo laparotomy in the 2nd or 3rd trimester. ConclusionsIn this large modern cohort, we found similar rates of bilaterality, torsion, malignant transformation, and struma ovarii in ovarian teratomas when compared to previous literature. Most cases of ovarian teratoma can be managed laparoscopically, which is associated with a lower surgical complication rate. Despite increased risk of cyst spillage with a minimally invasive approach, chemical peritonitis is a rare complication.

A311 EFFICACY OF PANCREATIC CANCER SCREENING IN HIGH RISK INDIVIDUALS

Abstract Background Pancreatic cancer (PC) is the 11th most prevalent cancer in Canada with about 6900 new cases in 2021. Due to late diagnosis, it is the 4th largest cause of cancer related mortality in Canada. Despite advances in diagnosis and treatment over the past decade, the 5-year survival remains ampersand:003C10%, making pancreatic cancer one of the most lethal malignancies worldwide. However, if detected early, the 5-year survival increases to 80%. The incidence of pancreatic cancer remains low. Therefore, the International Cancer of the Pancreas Screening (CAPS) consortium do not recommend screening everyone. Selective screening in high-risk individuals may be effective in early detection and treatment of pancreatic cancer. Aims 1. To determine the characteristics of patients undergoing PC screening at a single tertiary care center in Vancouver. 2. To determine the frequency of disease progression and outcomes in patients diagnosed with high-risk lesions during screening. Methods Retrospective chart review was performed for patients who were screened between January 1, 2005 to June 30, 2023. As per consensus from the CAPS Consortium, patients with at least 2 close relatives with PC, and those with predisposing genetic mutations screened using endoscopic ultrasound (EUS) and/or magnetic resonance imaging (MRI) were included in the study. Clinical data, such as age, tobacco use, genetic risk, endoscopic findings, and outcomes, such as disease progression, were collected. Statistical analysis was performed using Rstudio and Microsoft Excel. Results 73 patients were included, with 42 included due to pathogenic germline mutations, and 31 with familial pancreatic cancer. Most were female (52, 71.2%). Median age in the study was 54 years (range 24-74 years) at initial screening. 66 patients (90.4%) received an EUS and 7 patients (9.6%) received MRI for initial screening. At the time of initial screening. 74.6% of patients had a normal pancreas. 6 patients (8.2%) were found to have neoplastic lesions, e.g. ampulloma, neuroendocrine tumour or adenocarcinoma at initial screening and referred for surgery. In the median follow up time of 67.9 months (range 0-168.1 months), 6 patients were noted to have disease progression, 4 of which had increase in cyst size without any other worrisome features. 2 patients had progression to malignancy. 1 of those patients received chemotherapy and surgical resection and remains alive; the other patient was lost of follow up due to geographical move. Conclusions Pancreatic cancer screening in high risk individuals is effective in early detection and treatment. Given lower incidence of pancreatic cancer, we likely need additional patient enrolment to observe a significant improvement in patient outcomes. Funding Agencies None

Endoscopic endonasal marsupialization of rathke cleft cysts: clinical outcomes and risk factors analysis of visual impairment, pituitary dysfunction, and CSF leak.

This paper assesses the clinical and imaging characteristics, histopathological findings, and treatment outcomes of patients with Rathke's cleft cyst (RCC), as well as identifies potential risk factors for preoperative visual and pituitary dysfunction, intraoperative cerebrospinal fluid (CSF) leak, and recurrence. Through analyzing these factors, the study aims to contribute to the current understanding of the management of RCCs and identify opportunities for improving patient outcomes. We performed a retrospective analysis of 45 RCC patients between ages 18-80 treated by Endoscopic Endonasal Approach (EEA) and cyst marsupialization between 2010 and 2022 at a single institution. The median patient age was 34, and 73% were female. The mean follow-up was 70 ± 43 months. Preoperative visual impairment correlated with cyst diameter (OR = 1.41, 95% CI = 1.07 to 1.85, p-value = 0.01) and older age (OR = 1.06, 95% CI = 1.01 to 1.11, p-value = 0.02). Intraoperative CSF leaks were 11 times more likely for cysts ≥ 2cm (OR = 11.3, 95% CI = 1.25 to 97.37, p-value = 0.03), with the odds of leakage doubling for every 0.1cm increase in cyst size (OR = 1.41, 95% CI = 1.08 to 1.84, p-value = 0.01). Preoperative RCC appearing hypointense on T1 images demonstrated significantly higher CSF leak rates than hyperintense lesions (OR = 122.88, 95% CI = 1.5 to 10077.54, p-value = 0.03). Preoperative pituitary hypofunction was significantly more likely in patients with the presence of inflammation on histopathology (OR = 20.53, 95% CI = 2.20 to 191.45, p-value = 0.008 ) and T2 hyperintensity on magnetic resonance imaging (MRI) sequences (OR = 23.2, 95% CI = 2.56 to 211.02, p-value = 0.005). Notably, except for the hyperprolactinemia, no postoperative improvement was observed in pituitary function. Carefully considering risk factors, surgeons can appropriately counsel patients and deliver expectations for complications and long-term results. In contrast to preoperative visual impairment, preoperative pituitary dysfunction was found to have the least improvement post-surgery. It was the most significant permanent complication, with our data indicating the link to the cyst signal intensity on T2 MR and inflammation on histopathology. Earlier surgical intervention might improve the preservation of pituitary function.

Extracellular vesicles contribute to early cyst development in autosomal dominant polycystic kidney disease by cell-to-cell communication.

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the formation of fluid-filled cysts within the kidney due to mutations in PKD1 or PKD2. Although the disease remains incompletely understood, one of the factors associated with ADPKD progression is the release of nucleotides (including ATP), which can initiate autocrine or paracrine purinergic signaling by binding to their receptors. Recently, we and others have shown that increased extracellular vesicle (EVs) release from PKD1 knockout cells can stimulate cyst growth through effects on recipient cells. Given that EVs are an important communicator between different nephron segments, we hypothesize that EVs released from PKD1 knockout distal convoluted tubule (DCT) cells can stimulate cyst growth in the downstream collecting duct (CD). Here, we show that administration of EVs derived from Pkd1-/- mouse distal convoluted tubule (mDCT15) cells result in a significant increase in extracellular ATP release from Pkd1-/- mouse inner medullary collecting duct (iMCD3) cells. In addition, exposure of Pkd1-/- iMCD3 cells to EVs derived from Pkd1-/- mDCT15 cells led to an increase in the phosphorylation of the serine/threonine-specific protein Akt, suggesting activation of proliferative pathways. Finally, the exposure of iMCD3 Pkd1-/- cells to mDCT15 Pkd1-/- EVs increased cyst size in Matrigel. These findings indicate that EVs could be involved in intersegmental communication between the distal convoluted tubule and the collecting duct and potentially stimulate cyst growth.

A Combined DNA/RNA-based Next-Generation Sequencing Platform to Improve the Classification of Pancreatic Cysts and Early Detection of Pancreatic Cancer Arising From Pancreatic Cysts.

We report the development and validation of a combined DNA/RNA next-generation sequencing (NGS) platform to improve the evaluation of pancreatic cysts. Despite a multidisciplinary approach, pancreatic cyst classification, such as a cystic precursor neoplasm, and the detection of high-grade dysplasia and early adenocarcinoma (advanced neoplasia) can be challenging. NGS of preoperative pancreatic cyst fluid improves the clinical evaluation of pancreatic cysts, but the recent identification of novel genomic alterations necessitates the creation of a comprehensive panel and the development of a genomic classifier to integrate the complex molecular results. An updated and unique 74-gene DNA/RNA-targeted NGS panel (PancreaSeq Genomic Classifier) was created to evaluate 5 classes of genomic alterations to include gene mutations (e.g., KRAS, GNAS, etc.), gene fusions and gene expression. Further, CEA mRNA ( CEACAM5 ) was integrated into the assay using RT-qPCR. Separate multi-institutional cohorts for training (n=108) and validation (n=77) were tested, and diagnostic performance was compared to clinical, imaging, cytopathologic, and guideline data. Upon creation of a genomic classifier system, PancreaSeq GC yielded a 95% sensitivity and 100% specificity for a cystic precursor neoplasm, and the sensitivity and specificity for advanced neoplasia were 82% and 100%, respectively. Associated symptoms, cyst size, duct dilatation, a mural nodule, increasing cyst size, and malignant cytopathology had lower sensitivities (41-59%) and lower specificities (56-96%) for advanced neoplasia. This test also increased the sensitivity of current pancreatic cyst guidelines (IAP/Fukuoka and AGA) by >10% and maintained their inherent specificity. PancreaSeq GC was not only accurate in predicting pancreatic cyst type and advanced neoplasia but also improved the sensitivity of current pancreatic cyst guidelines.

Immune recognition of latent neuronal Toxoplasma gondiiinfection

Abstract Toxoplasma gondii is a neurotropic parasite with high mortality rates in immunocompromised individuals. Successful therapies have been developed against the acute tachyzoite stage, but no treatment options exist to target the latent form of the parasite – bradyzoites that form cysts within neurons. It is thought that residence of cysts in neurons shelters T. gondii from immune recognition and clearance. However, a decline in cyst numbers over time suggests the presence of anti-cyst immunity. Additionally, neuronal deletion of STAT1 results in increased cyst size and number, but no change in tachyzoite replication, providing evidence for IFN-γ-mediated cyst control. T cells are a major source of IFN-γ and are crucial for parasite control in the CNS, but it remains unclear if T cells directly target cyst-infected neurons. The use of transgenic parasites with bradyzoite-restricted OVA expression (BagOVA) reveals that cyst-derived antigen can induce OT-I CD8+ T cell responses in the brains of chronically infected mice. These cyst-specific OT-Is have distinct effector phenotypes and cytokine production when compared to T cells responding to antigen expressed by both tachyzoites and bradyzoites (ptubOVA). To determine whether neurons directly present cyst antigen to induce CD8+ T cell responses in BagOVA infection, mice with neuronal-specific loss of MHCI were generated. Infection of these mice showed no significant change in the magnitude of T cell responses or parasite burden in the CNS. Together these findings reveal that T cell responses can be mounted against cyst antigens, but another cell type likely mediates cross presentation of cyst derived antigens. Supported by grants from NIH (R01 580-5803-4-580697-XXXX-2000-3000)

Effect of within-endobag method during laparoscopic ovarian cystectomy of dermoid cyst: A retrospective study.

This study aimed to evaluate the effect of within-endobag cystectomy during and after laparoscopic ovarian cystectomy in patients with dermoid cysts. We retrospectively analyzed 84 patients with ovarian dermoid cysts who underwent laparoscopic ovarian cystectomy. In 30 patients, the affected ovary was placed in an endobag before cystectomy and cystectomy was performed within an endobag (within-endobag group), while the remaining 54 patients underwent standard cystectomy without this step (without-endobag group). After cystectomy, the cyst wall was placed in an endobag and was removed from the abdomen. Compared with the without-endobag group, the within-endobag group had a significantly lower rate of cyst content spillage (23.3% vs 72.2%, P < .001) and significantly shorter operation times when the cysts ruptured (23.4 ± 8.6 minutes vs 51.2 ± 28.6 minutes, P < .001). Whereas there was no significant difference in operation time in the absence of cyst rupture between 2 groups (21.2 ± 8.8 minutes vs 31.1 ± 17.4 minutes, P = .111). In patients with cyst rupture, according to the cyst size increase, the operation time was significantly prolonged without-endobag, whereas no significant prolongation was observed in within-endobag cystectomy. Except for operation time, there were no significant differences in cyst length, pain on the first day after surgery, hemoglobin loss, hospital stay, and inflammatory markers (C-reactive protein and white blood cell counts) in both ruptured and unruptured cases between the 2 groups. There were no postoperative complications in the within-endobag group, but 2 cases of perioperative complications occurred in the without-endobag group. No chemical peritonitis due to spillage of the cyst contents was observed in either group. Laparoscopic ovarian cystectomy performed within-endobag can reduce both the spillage rate of cyst contents and operation time regardless of cyst size in patients with ruptured cysts. Therefore, this technique is a good surgical option for the laparoscopic ovarian cystectomy of large dermoid ovarian cysts.

LBODP032 Case Report Of A 13 Years Old Female With A Novel Pathogenic Variant In The Hnf1b Gene

Abstract Background MODY5 results from mutations in the gene encoding hepatocyte nuclear factor 1 homeobox B (HNF1B), a transcription factor critical in development of the pancreas, liver, intestines, and the urogenital tract (1). We report the case of a pediatric patient with a novel frameshift variant (p. Phe309SerfsTer18) in the HNF1B gene accounting for the co-occurrence of diabetes mellitus and structural renal abnormalities. Clinical case: A 13-years-old female with chronic kidney disease stage 3 (eGFR = 48 ml/min/1.73m2) secondary to congenital dysplastic-appearing left kidney and surgical removal of a cystic dysplastic right kidney was referred to our diabetes clinic for hyperglycemia. She reported long-standing polyuria and polydipsia previously attributed to her kidney disease. She required laparoscopic right nephrectomy at age 5 years due to increasing cyst size. Her left kidney also has small renal parenchymal cysts which have been monitored over time. There is no known family history of kidney disease, and grandparents have a history of diabetes. Laboratory investigation revealed a random blood glucose of 478 mg/dL and a hemoglobin A1c of 12.2%. She was not in diabetic ketoacidosis. She had a detectable insulin level of 5.1 mcIU/mL and C-peptide was 3.4 ng/mL. She was initially presumed to have Type 1 diabetes based on a relatively low insulin level and started on basal-bolus insulin (up to 0.59 units/kg of weight/day). Glycosylated hemoglobin was 8.7% one month after diagnosis. Glutamic acid decarboxylase, zinc transporter, islet cell, and insulin autoantibodies were not detected, however, and she was then suspected to have either Type 2 diabetes or MODY. Approximately one year later, she was taken off insulin due to hypoglycemia and managed only with metformin 500 mg twice daily. Next-generation sequencing revealed a de novo pathogenic variant in HNF1B causing a frameshift deletion (p. Phe309SerfsTer18). Genetic testing of parents was negative. Once her diagnosis of MODY5 was made, she was started on glipizide 2.5 mg once daily which has been titrated up to a dose of 5 mg with some success. However, she has since been re-started on insulin (up to 0.2 units/kg/day) due to hyperglycemia keeping in line with the MODY5 phenotype likely causing destruction of beta cells over time (1). Conclusion We report a case of MODY5 with a novel frameshift mutation (p. Phe309SerfsTer18) in the HNF1B gene. This case highlights the importance of screening for HNF-1B mutations in those patients with structural renal abnormalities and hyperglycemia, as diagnosis can guide management decisions and provide prognostic information. Reference Bellanné-Chantelot C, Chauveau D, Gautier JF, et al. Clinical spectrum associated with hepatocyte nuclear factor-1beta mutations. Ann Intern Med. 2004;140(7): 510-517. doi: 10.7326/0003-4819-140-7-200404060-0000 Presentation: No date and time listed

Systematically quantifying morphological features reveals constraints on organoid phenotypes.

Organoids recapitulate complex 3D organ structures and represent a unique opportunity to probe the principles of self-organization. While we can alter an organoid's morphology by manipulating the culture conditions, the morphology of an organoid often resembles that of its original organ, suggesting that organoid morphologies are governed by a set of tissue-specific constraints. Here, we establish a framework to identify constraints on an organoid's morphological features by quantifying them from microscopy images of organoids exposed to a range of perturbations. We apply this framework to Madin-Darby canine kidney cysts and show that they obey a number of constraints taking the form of scaling relationships or caps on certain parameters. For example, we found that the number, but not size, of cells increases with increasing cyst size. We also find that these constraints vary with cyst age and can be altered by varying the culture conditions. We observed similar sets of constraints in intestinal organoids. This quantitative framework for identifying constraints on organoid morphologies may inform future efforts to engineer organoids.

Characteristics of ovarian endometrioma during pregnancy

During pregnancy, the ovarian endometrioma generally decreases in size and occasionally ruptures. We evaluated (1) whether and how ovarian-endometrioma size changes from the first trimester to the postdelivery period, and (2) the type of endometrioma more likely to rupture during pregnancy. During an 18-year period (2000-2018), ultrasound in the first trimester revealed ovarian endometrioma in 149 pregnant women at our tertiary institute. Among these, we subjected 138 endometriomas in 145 patients to expectant management (wait-and-watch approach during pregnancy). We compared the cyst sizes in the first trimester and the postdelivery period, and defined a >1cm diameter size-change as a significant increase/decrease. We analyzed four patients with rupture and characterized the predictors of rupture. A comparison of cyst sizes in the first trimester and the postdelivery period revealed that the size of 94 (68%), 37 (27%), and 7 ovaries (5.0%), respectively, decreased, remained unchanged, and increased; in 56 ovaries (40%), apparent cysts were no longer present. Of the 145 patients, four (2.8%) required emergency surgery for cyst rupture. Adhesion to the surroundings, an increase in cyst size, large size (diameter of ≥6cm), and compression due to the enlarged uterus in late pregnancy were factors clinically related to rupture. Approximately two-thirds of ovarian endometriomas decreased in size during pregnancy (40% disappeared), 27% remained unchanged, and only 5% increased in size. However, 2.8% of pregnant women with endometrial cysts experienced rupture. We characterized risk factors for rupture; however, clinical application requires further evaluation.

Clinical significance of incidentally discovered renal cysts in pediatric patients.

To determine the clinical significance of incidentally discovered renal cysts in pediatric patients and identify imaging predictors of autosomal dominant polycystic kidney disease (ADPKD). A retrospective search of radiology reports from 2000 to 2016 was performed to identify patients < 18years old with an imaging exam identifying at least one renal cyst and a ≥ 1-year follow-up renal imaging exam for cyst evaluation and/or diagnosis of ADPKD. Cysts with clear solid mass components were excluded. 84 pediatric patients with renal cysts were identified (mean age, 9.5 years), including 76 patients with incidentally discovered cysts and 8 patients with cysts identified from screening for ADPKD family history. Among the incidentally discovered cyst group, 7.9% were found to have ADPKD compared with 100% of patients with cysts and ADPKD family history. Maximum cyst diameter was significantly increased in patients with ADPKD compared to patients without ADPKD (22.0mm vs 12.7mm; P < 0.001, Fisher's Exact test). Multiple cysts or bilateral cysts were imaging features associated with a significantly higher (P < 0.01) incidence of ADPKD, both for the entire study population and the incidentally discovered cyst group. An increase in cyst size on the follow-up study was associated with higher incidence of ADPKD (P < 0.05). No malignancies were identified. Incidentally discovered renal cysts in pediatric patients are associated with a small but non-zero risk of ADPKD. Among cyst characteristics, bilaterality, multiplicity, large size, and increased size on follow-up imaging were associated with a statistically significant elevation of ADPKD risk, and should prompt diagnostic evaluation.

Impact of endoscopic ultrasound on diagnosis and management of presumed mucinous neoplasms when done for pancreatic cyst morphology change on non-invasive surveillance imaging.

Background and study aims Guidelines for management of presumed neoplastic pancreatic cysts have encouraged noninvasive imaging for low-risk surveillance, while reserving endoscopic ultrasound for worrisome features including morphologic change. We aim to study the impact of endoscopic ultrasound on diagnosis and management compared with non-invasive imaging. Patients and methods A single-institution pancreatic cyst database was retrospectively queried for patients who underwent endoscopic ultrasound for the indication of change in cyst morphology. Diagnoses were classified as presumed mucinous neoplasm with or without worrisome features or high-risk stigmata and non-mucinous lesions. Management decisions were defined a priori as surgical evaluation for patients with high-risk stigmata, positive cytology or mural nodule, or continued surveillance for all others. Results Between January 2013 and October 2016, 709 pancreas cyst endoscopic ultrasounds were performed of which 89 were for cyst morphology change seen on noninvasive imaging including 10 presumed pseudocysts, nine presumed serous cystadenomas, and 70 presumed mucinous cystic neoplasms. Cyst morphologic changes included increase in caliber of the main pancreatic duct (7 cases), increase in cyst size (68 cases), cyst ≥ 30 mm (10 cases), and presence of a solid nodule (1 case). Median cyst size increase was 5 mm with interquartile range of 4 mm over 2.1 ± 1.9 years. Endoscopic ultrasound done for morphologic change resulted in a change in diagnosis and management in 16 % and 13 % of cases, respectively. Conclusion Endoscopic ultrasound has a modest but clinically significant role in impacting diagnosis and management for presumed mucinous cystic neoplasms when performed for the indication of cyst morphology change.

NHA2 promotes cyst development in an invitro model of polycystic kidney disease.

Significant and selective up-regulation of the Na+ /H+ exchanger NHA2 (SLC9B2) was observed in cysts of patients with autosomal dominant polycystic kidney disease. Using the MDCK cell model of cystogenesis, it was found that NHA2 increases cyst size. Silencing or pharmacological inhibition of NHA2 inhibits cyst formation invitro. Polycystin-1 represses NHA2 expression via Ca2+ /NFAT signalling whereas the dominant negative membrane-anchored C-terminal fragment (PC1-MAT) increased NHA2 levels. Drugs (caffeine, theophylline) and hormones (vasopressin, aldosterone) known to exacerbate cysts elicit NHA2 expression. Taken together, the findings reveal NHA2 as a potential new player in salt and water homeostasis in the kidney and in the pathogenesis of polycystic kidney disease. Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 and PKD2 encoding polycystin-1 (PC1) and polycystin-2 (PC2), respectively. The molecular pathways linking polycystins to cyst development in ADPKD are still unclear. Intracystic fluid secretion via ion transporters and channels plays a crucial role in cyst expansion in ADPKD. Unexpectedly, we observed significant and selective up-regulation of NHA2, a member of the SLC9B family of Na+ /H+ exchangers, that correlated with cyst size and disease severity in ADPKD patients. Using three-dimensional cultures of MDCK cells to model cystogenesis invitro, we showed that ectopic expression of NHA2 is causal to increased cyst size. Induction of PC1 in MDCK cells inhibited NHA2 expression with concordant inhibition of Ca2+ influx through store-dependent and -independent pathways, whereas reciprocal activation of Ca2+ influx by the dominant negative membrane-anchored C-terminal tail fragment of PC1 elevated NHA2. We showed that NHA2 is a target of Ca2+ /NFAT signalling and is transcriptionally induced by methylxanthine drugs such as caffeine and theophylline, which are contraindicated in ADPKD patients. Finally, we observed robust induction of NHA2 by vasopressin, which is physiologically consistent with increased levels of circulating vasopressin and up-regulation of vasopressin V2 receptors in ADPKD. Our findings have mechanistic implications on the emerging use of vasopressin V2 receptor antagonists such as tolvaptan as safe and effective therapy for polycystic kidney disease and reveal a potential new regulator of transepithelial salt and water transport in the kidney.

Downstream Costs Associated With Incidental Pancreatic Cysts Detected at MRI.

The purpose of this study is to assess downstream costs associated with pancreatic cysts incidentally detected at MRI. Two hundred patients with an incidental pancreatic cyst detected at MRI were identified. Downstream events (imaging, office visits, endoscopic ultrasound-guided fine-needle aspiration, or chemotherapy) were identified from the electronic medical record. Radiologists' recommendations and ordering physician management were classified relative to the American College of Radiology (ACR) incidental findings committee recommendations. Costs for the downstream events were estimated using national Medicare rates and a 3% annual discount rate. Mean costs were computed. Estimated downstream costs averaged $460 per cyst ($872 per cyst with any follow-up testing). Nine patients had a clinically relevant outcome during follow-up (increase in cyst size, development of new cyst, or development of pancreatic cancer). Downstream cost per cyst with a clinically relevant outcome was $1364. Costs were greater when ordering physicians overmanaged ($842) versus when they were adherent ($631) or undermanaged ($252) relative to radiologist recommendation. Although costs were $252 when ordering physicians undermanaged relative to ACR incidental findings committee recommendations, costs were similar when ordering physicians were adherent ($811) or overmanaged ($845) relative to ACR incidental findings committee recommendations. Costs did not vary significantly according to whether radiologists recommended follow-up testing ($317-$491) or whether radiologist recommendations were adherent, undermanaged, or overmanaged relative to ACR incidental findings committee recommendations ($344-$528). The findings suggest a role for targeted educational efforts, collaborative partnerships, and other initiatives to foster greater adherence to radiologist recommendations, including critical test results notification systems, automated reminders within electronic health systems, and stronger language within radiology reports when no follow-up testing is recommended.

When to Consider an Increase in Cyst Size in Patients With Pancreatic Cystic Neoplasms?

Introduction: Because of the interobserver variability in radiographic cyst size evaluation, a pancreatic cyst growth rate of ≥ 3mm/year warrants a shorter follow up interval by the current consensus. On the other hand, a 20% increase in the maximum diameter of the cyst size compared to the previous was also considered to be a significant interval change by the existing literature. We aim to determine the significance of an increase in cyst size of ≥ 3mm versus ≥ twenty percent with respect to the initial cyst size in patients with pancreatic cystic neoplasms (PCN). Methods: From the Mayo Clinic, Rochester database, we retrospectively identified 238 subjects with incidentally detected pancreatic cysts. We excluded the patients with inflammatory fluid collections secondary to acute pancreatitis. The study subjects were further classified into three groups according to the initial cyst size of <1cm (99 patients), 1-3 cm (124 patients), and ≥3cm (15 patients) respectively. A difference in 3mm versus twenty percent increase at follow-up from the initial cyst size was compared in each group. Results: Baseline characteristics of the included patients were summarized in the Table. The mean cyst size at baseline was 13.3±9.4 mm which were followed up for a mean duration of 95.5±29.2 months. During the follow up period, out of 238 patients, 104 patients showed ≥3mm cyst growth compared to 110 patients with ≥20% increase in cyst size. In patients with smaller cysts (<1cm), a higher proportion of patients (56.4% versus 47.1%, p < 0.0001) showed a twenty percent difference in cyst size compared to 3mm cyst growth [Figure]. Whereas in patients with cysts size ≥1cm, higher number of patients (48.1% versus 40%; p < 0.0001 for 1-3cm, 4.8% versus 3.6%; p = 0.004 for ≥ 3cm cyst size) showed a cyst growth of 3mm compared to twenty percent increase in cyst size. Conclusion: For any cyst size, an increase in 20% of the maximum diameter of the cyst size is reasonable to consider as a significant change to reduce the effect of measuring error.61_A Figure 1. Baseline characteristics of the patients61_B Figure 2. Proportion of patients with 3mm versus 20% cyst growth with respect to the baseline cyst size

Endoscopic treatment of intraventricular ependymal cysts in children: personal experience and review of literature.

Intracranial ependymal cysts are rare neuroepithelial cysts that occur less frequently than arachnoid cysts. The cysts are most often intraparenchymal, but they are rarely reported to be intraventricular. This study evaluates the role of endoscopy in the treatment of intraventricular ependymal cysts (IVECs). Twelve pediatric patients (mean age 4.3years) with symptomatic IVECs were the subject of this study. The cyst was located inside the lateral ventricle in all cases (100%), it was present in trigone (10 patients, 83.3%), and in temporal horn (2 patients, 16.7%). Concomitant hydrocephalus was present in two patients (16.7%). All patients underwent operations through a purely endoscopic procedure. Communication of the cyst with the subarachnoid space was performed in six patients (50%); endoscopic cystocisternostomy was performed in four patients (33.3%), and endoscopic cystoventriculostomy in two patients (16.7%). Postoperative clinical improvement associated with postoperative reduction in cyst size was encountered in ten patients (83.3%). Improvement of hydrocephalus occurred in both patients who had hydrocephalus (100%). There were no deaths or permanent morbidity. Among the follow-up period (mean 44.3months), none of the patients required a repeat endoscopic procedure due to recurrence of symptoms or increase in cyst size. Intraventricular ependymal cysts can be effectively treated by endoscopy. Endoscopic fenestration of the cyst wall into subarachnoid space, basal cisterns, or ventricular system can be used in the treatment of these patients with postoperative symptomatic improvement and reduction of cyst size. The procedure is simple, effective, minimally invasive, and associated with low morbidity and mortality rates.

Issue Highlights

Issue Highlights

Longitudinal changes in magnetic resonance imaging of malignant and borderline tumors associated with ovarian endometriotic cyst comparing with endometriotic cysts without arising malignancy

PurposeTo investigate magnetic resonance (MR) findings and to detect malignant transformation of ovarian endometriotic cysts by comparing longitudinal changes in patients with ovarian malignant/borderline tumors associated with ovarian endometriotic cysts (tumor group) with those of patients with endometriotic cysts (control group). MethodsTumor group patients (n = 10) had ovarian malignant/borderline tumors with pathologically confirmed association with endometriosis and available prior MRI of endometriotic cysts. Control group patients (n = 40) had been diagnosed more than two times as having ovarian endometriotic cysts by MRI examination. The tumor and solid portion sizes were measured. Two radiologists independently evaluated signal intensity (SI) of the cystic portion on both T1-weighted and T2-weighted images (WI), presence of shading on T2WI, and T2 dark spot sign in both groups and evaluate longitudinal changes of those findings. ResultsPathological diagnoses of the tumor group were clear cell carcinoma (n = 6), endometrioid carcinoma (n = 1), serous carcinoma (n = 1), mucinous borderline tumor (n = 1), and endometrioid borderline tumor (n = 1). Tumor size had increased significantly in the tumor group (p = .004), but not in controls. Solid portions were identified in all cases only when neoplasms were suspected. Disappearance of shading during the follow-up period was observed more in tumor group (n = 2) than in the controls (n = 0). No significant difference was found between groups in the SI on T1 and T2WI, and T2 dark spot sign for the two MR examinations. ConclusionsThe MR findings suggesting malignant transformation were emergence of a solid portion and increase in cyst size. Disappearance of shading also facilitates the follow-up of endometriotic cysts.